Abstract

ObjectiveWhile frozen section methods have been widely conducted to determine aganglionosis segment during transanal endorectal pull-through (TEPT) for Hirschsprung disease (HSCR) patients in most institutions, some hospitals still rely on contrast enema to predict aganglionosis segments due to unavailability of frozen section facilities. We determined the accuracy of transition zone in contrast enema to predict aganglionosis segments during TEPT. We retrospectively reviewed all contrast enema and frozen sections for HSCR patients under 2 years of age who underwent TEPT at our institution.ResultsWe recruited 36 HSCR patients: twenty-six patients (72.2%) had radiographic transition zones limited to rectum, while ten subjects (27.8%) were limited to rectosigmoid. The rectum subgroup of patients showed a concordance of 30.8%, whereas the rectosigmoid subgroup had a concordance of 100%. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of contrast enema compared with intraoperative histopathological findings for aganglionosis level were 100% (95% CI 0.60–1.0), 35.7% (95% CI 0.19–0.56), 30.8% (95% CI 0.15–0.52), 100% (95% CI 0.66–1.0), and 50% (95% CI 0.33–0.67), respectively. In conclusions, contrast enema has low accuracy to predict intraoperative aganglionosis segments in HSCR patients, indicating that it might not be utilized to determine aganglionosis level during TEPT.

Highlights

  • Hirschsprung disease (HSCR) is a neurodevelopmental disorder characterized by the lack of ganglion cells in the bowel, resulting in functional obstruction in infants [1–3]

  • While frozen section methods have been widely conducted for intraoperative evaluation to determine the aganglionosis segment during transanal endorectal pull-through (TEPT) in most pediatric surgical centers [7–10], there are some hospitals that still rely on contrast enema to predict aganglionosis segment preoperatively due to unavailability of frozen section facilities

  • The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy rates of the contrast enema compared with intraoperative histopathological findings for aganglionosis level were 100%, 35.7%, 30.8%, 100%, and 50%, respectively (Table 2)

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Summary

Results

We identified 47 patients with HSCR during a 2-year period of study. We excluded 11 HSCR patients because of no contrast enema available, we further analyzed 36 HSCR patients, consisting of 18 males and 18 females. Twenty-six patients (72.2%) had radiographic transition zones limited to the rectum, while ten subjects (27.8%) were limited to the rectosigmoid (Table 1). The. Age at TEPT 1–6 months old 7–12 months old Contrast enema transition zone Rectum Rectosigmoid. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy rates of the contrast enema compared with intraoperative histopathological findings for aganglionosis level were 100% (95% CI 0.60–1.0), 35.7% (95% CI 0.19–0.56), 30.8% (95% CI 0.15–0.52), 100% (95% CI 0.66–1.0), and 50% (95% CI 0.33–0.67), respectively (Table 2). We determined the Cohen’s Kappa index for the contrast enema transition zone and the aganglionosis level of intraoperative histopathological findings, showing its index of 0.198 (slight agreement), while the McNemar test revealed that the sensitivity and specificity rates was significantly different between contrast enema and intraoperative histopathological findings (p = 0.0001)

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